{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,26]],"date-time":"2026-06-26T22:44:08Z","timestamp":1782513848259,"version":"3.54.5"},"reference-count":5,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2015,6,25]],"date-time":"2015-06-25T00:00:00Z","timestamp":1435190400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2015,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Mast cell (<jats:styled-content style=\"fixed-case\">MC<\/jats:styled-content>) activation syndrome (<jats:styled-content style=\"fixed-case\">MCAS<\/jats:styled-content>) is a recently recognized, likely prevalent collection of heterogeneous illnesses of inappropriate <jats:styled-content style=\"fixed-case\">MC<\/jats:styled-content> activation with little to no <jats:styled-content style=\"fixed-case\">MC<\/jats:styled-content> neoplasia likely driven by heterogeneous patterns of constitutively activating mutations in <jats:styled-content style=\"fixed-case\">MC<\/jats:styled-content> regulatory elements including various tyrosine kinases (<jats:styled-content style=\"fixed-case\">TK<\/jats:styled-content>s, dominantly <jats:styled-content style=\"fixed-case\">KIT<\/jats:styled-content>). <jats:styled-content style=\"fixed-case\">MCAS<\/jats:styled-content> typically presents as chronic multisystem polymorbidity of generally inflammatory\u00a0\u00b1\u00a0allergic theme. As with indolent systemic mastocytosis (<jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>), treatment of <jats:styled-content style=\"fixed-case\">MCAS<\/jats:styled-content> focuses more against <jats:styled-content style=\"fixed-case\">MC<\/jats:styled-content> mediators than <jats:styled-content style=\"fixed-case\">MC<\/jats:styled-content> neoplasia, but some cases prove refractory even to the <jats:styled-content style=\"fixed-case\">TK<\/jats:styled-content> inhibitor (<jats:styled-content style=\"fixed-case\">TKI<\/jats:styled-content>) imatinib reported useful both in uncommon <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> cases not bearing <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>'s usual imatinib\u2010resistant <jats:styled-content style=\"fixed-case\">KIT<\/jats:styled-content>\u2010D816V mutation and in some cases of <jats:styled-content style=\"fixed-case\">MCAS<\/jats:styled-content> (which rarely bears <jats:styled-content style=\"fixed-case\">KIT<\/jats:styled-content>\u2010D816V). Most allergy is principally a <jats:styled-content style=\"fixed-case\">MC<\/jats:styled-content> activation phenomenon and sunitinib is a multitargeted <jats:styled-content style=\"fixed-case\">TKI<\/jats:styled-content> shown helpful in controlling a murine model of oral allergy syndrome. We present the first report of use of sunitinib in life\u2010threatening <jats:styled-content style=\"fixed-case\">MCAS<\/jats:styled-content> refractory to multiple agents including imatinib achieving immediate, complete, sustained, non\u2010toxic remission suggesting a new option for treatment of aggressive <jats:styled-content style=\"fixed-case\">MC<\/jats:styled-content> disease.<\/jats:p>","DOI":"10.1111\/ejh.12606","type":"journal-article","created":{"date-parts":[[2015,6,13]],"date-time":"2015-06-13T07:01:41Z","timestamp":1434178901000},"page":"595-597","source":"Crossref","is-referenced-by-count":14,"title":["Successful treatment of mast cell activation syndrome with sunitinib"],"prefix":"10.1111","volume":"95","author":[{"given":"Lawrence B.","family":"Afrin","sequence":"first","affiliation":[{"name":"Division of Hematology, Oncology, and Transplantation University of Minnesota Minneapolis MN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank M.","family":"Cichocki","sequence":"additional","affiliation":[{"name":"Division of Hematology, Oncology, and Transplantation University of Minnesota Minneapolis MN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kamal","family":"Patel","sequence":"additional","affiliation":[{"name":"CARTI Hematology\/Oncology Little Rock AR USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerhard J.","family":"Molderings","sequence":"additional","affiliation":[{"name":"Institute for Human Genetics University of Bonn Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2015,6,25]]},"reference":[{"key":"e_1_2_8_2_1","first-page":"146","article-title":"The presentation, diagnosis and treatment of mast cell activation syndrome","volume":"27","author":"Afrin LB","year":"2014","journal-title":"Curr Allergy Clin Immunol"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.critrevonc.2014.09.001"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00726-010-0486-6"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1517\/13543784.2013.740010"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1398-9995.2011.02717.x"}],"container-title":["European Journal of Haematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fejh.12606","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ejh.12606","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,3]],"date-time":"2023-10-03T19:29:09Z","timestamp":1696361349000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ejh.12606"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,6,25]]},"references-count":5,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2015,12]]}},"alternative-id":["10.1111\/ejh.12606"],"URL":"https:\/\/doi.org\/10.1111\/ejh.12606","archive":["Portico"],"relation":{},"ISSN":["0902-4441","1600-0609"],"issn-type":[{"value":"0902-4441","type":"print"},{"value":"1600-0609","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,6,25]]}}}